Intestinal necrosis from congenital hypercoagulopathy

Protein-S deficiency is a rare congenital disorder that results in a hypercoagulable state, typically presenting as recurrent venous thrombosis in adults. Few cases have been reported in children. A 2-year-old girl presented in septic shock. During laparotomy, ischemic necrosis of the intestine was noted, which extended from the gastroesophageal junction to the midjejunum. Total gastrectomy, pancreaticoduodenectomy, and partial resection of the small intestine were performed. She was left with 140 cm of distal small bowel, and had reconstruction with a single limb for the pancreatico-jejunostomy, choledochojejunostomy, and esophagojejunostomy to preserve intestinal length. Postoperative evaluation eventually disclosed a previously unrecognized protein-S deficiency. The hypercoagulopathy of protein-S deficiency can be complicated by catastrophic thrombotic events that require surgery. This congenital disorder should be considered in any child who has an unexplained thrombotic episode, because failure to recognize protein-S deficiency may have potentially fatal consequences.